Renal - Congenital Abnormalities; Acute Renal Failure Flashcards

1
Q

What is the most common congenital renal anomaly?

A

Horseshoe kidney

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2
Q

Horseshoe kidneys are typically conjoined at the _______ renal ______.

A

Horseshoe kidneys are typically conjoined at the lower renal pole.

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3
Q

In its ascent from the pelvis, horseshoe kidneys most commonly get caught where / on what?

A

The inferior mesenteric artery

(IMA root)

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4
Q

Although horseshoe kidneys are typically ______, there may be some increased risk of certain ______s.

A

Although horseshoe kidneys are typically benign, there may be some increased risk of certain malignancies.

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5
Q

Renal _______ refers to the absence of one or both kidneys.

A

Renal agenesis refers to the absence of one or both kidneys.

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6
Q

Unilateral renal agenesis leads to ___________ of the existing kidney; hyperfiltration leads to increased risk of ______ ______ later in life.

A

Unilateral renal agenesis leads to hypertrophy of the existing kidney; hyperfiltration leads to increased risk of renal failure later in life.

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7
Q

Bilateral renal agenesis leads to what condition(s)?

A

Potter’s syndrome

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8
Q

What noninherited, congenital malformation of the renal parenchyma is characterized by cysts and abnormal tissue?

A

Dysplastic kidney

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9
Q

Dysplastic kidneys are usually ____lateral.

A

Dysplastic kidneys are usually unilateral.

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10
Q

When bilateral renal cysts are present, polycystic kidney disease must be differentiated from what?

A

Dysplastic kidneys

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11
Q

Polycystic kidney disease leads to bilateral enlarged kidneys with cysts in which: the renal cortex or the renal medulla?

A

Both

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12
Q

Autosomal dominant cases of polycystic kidney disease are associated with mutations in what two genes?

A

(1) APKD1
(2) APKD2

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13
Q

Autosomal dominant polycystic kidney disease typically presents first in __________ (age group) as worsening renal failure, hypertension, and hematuria.

A

Autosomal dominant polycystic kidney disease typically presents first in young adults as worsening renal failure, hypertension, and hematuria.

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14
Q

Autosomal recessive polycystic kidney disease typically presents first in __________ (age group) as worsening renal failure, hypertension, and hematuria.

A

Autosomal recessive polycystic kidney disease typically presents first in infants as worsening renal failure and hypertension.

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15
Q

Autosomal _________ polycystic kidney disease typically presents in young adults as worsening renal failure, hypertension, and hematuria.

A

Autosomal dominant polycystic kidney disease typically presents in young adults as worsening renal failure, hypertension, and hematuria.

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16
Q

Autosomal _________ polycystic kidney disease typically presents in infants as worsening renal failure and hypertension.

A

Autosomal recessive polycystic kidney disease typically presents in infants as worsening renal failure and hypertension.

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17
Q

True/False.

Newborns with autosomal recessive polycystic kidney disease can present with Potter’s syndrome.

A

True.

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18
Q

Is autosomal dominant polycystic kidney disease associated with high- or low-renin hypertension?

A

High-renin

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19
Q

What are the main presenting S/Sy of a patient with autosomal dominant polycystic kidney disease?

A

Hypertension

Worsening renal failure

Hematuria

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20
Q

Autosomal recessive kidney disease is associated with major damage to what organ besides the kidneys?

A

The liver

(associated with hepatic fibrosis and hepatic cysts)

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21
Q

Autosomal dominant kidney disease is associated with what extra-renal pathologies?

A

Berry aneurysms;

hepatic cysts;

mitral valve prolapse

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22
Q

A newborn presents with hypertension, portal hypertension, and markedly elevated creatinine; exam shows bilateral renal cysts and hepatic fibrosis.

What is the diagnosis?

A

Autosomal recessive polycystic kidney disease

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23
Q

What autosomal dominant disease results in atrophic kidneys, worsening renal failure, and medullary (but not cortical) renal cysts?

A

Medullary cystic kidney disease

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24
Q

Medullary cystic kidney disease leads to atrophic kidneys with cysts in which: the renal cortex or the renal medulla or both?

A

The medulla only

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25
Q

Identify which of the following congenital renal abnormalities results in worsening renal failure:

Autosomal dominant polycystic kidney disease

Autosomal recessive polycystic kidney disease

Medullary cystic kidney disease

A

All three

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26
Q

Acute renal failure is a severe _________ in kidney function that develops over a period of a few _____s.

A

Acute renal failure is a severe decrease in kidney function that develops over a period of a few days.

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27
Q

The hallmarks of acute renal failure include: ______emia, increased _______ and _______ (lab values), and (often) ____uria.

A

The hallmarks of acute renal failure include: azotemia, increased BUN and creatinine, and oliguria.

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28
Q

A patient presents with azotemia, increased BUN and creatinine, and oliguria. Her S/Sy have been increasing rapidly over the past few days.

What is the immediate diagnosis?

A

Acute renal failure

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29
Q

Acute renal failure can be divided into what three forms of azotemia (based on etiology)?

A

Prerenal

Intrarenal

Postrenal

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30
Q

Prerenal azotemia is a form of acute renal failure that most often occurs due to decreased what?

A

Renal blood flow

(e.g. due to cardiac failure)

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31
Q

Prerenal azotemia is a form of acute renal failure that often occurs due to __________ GFR.

A

Prerenal azotemia is a form of acute renal failure that often occurs due to decreased GFR.

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32
Q

Is tubular function increased, decreased, or preserved in cases of acute renal failure due to prerenal azotemia?

A

Preserved

(unchanged)

33
Q

In cases of prerenal azotemia, serum BUN:creatinine > _____.

A

In cases of prerenal azotemia, serum BUN:creatinine > 15.

34
Q

In cases of prerenal azotemia, tubular function is preserved;

FENa < ____% and osmolarityurine > ____ mOsm/kg.

A

In cases of prerenal azotemia, tubular function is preserved;

FENa < 1% and osmolarityurine > 500 mOsm/kg.

35
Q

Postrenal azotemia is a form of acute renal failure that most often occurs due to what?

A

Urinary tract obstruction

(downstream from the kidney; e.g. ureter obstruction)

36
Q

Postrenal azotemia is a form of acute renal failure that often results in a __________ GFR.

A

Postrenal azotemia is a form of acute renal failure that often results in a decreased GFR.

37
Q

Is tubular function increased, decreased, or preserved in the early cases of acute renal failure due to postrenal azotemia?

A

Preserved

(unchanged)

38
Q

In early cases of postrenal azotemia, serum BUN:creatinine ___ 15.

A

In early cases of postrenal azotemia, serum BUN:creatinine > 15.

39
Q

In early cases of postrenal azotemia, tubular function is preserved;

FENa < ____% and osmolarityurine > ____ mOsm/kg.

A

In early cases of postrenal azotemia, tubular function is preserved;

FENa < 1% and osmolarityurine > 500 mOsm/kg.

40
Q

Is tubular function increased, decreased, or preserved in late cases of acute renal failure due to postrenal azotemia?

A

Decreased

(due to tubular damage)

41
Q

In late cases of postrenal azotemia, serum BUN:creatinine ___ 15.

A

In late cases of postrenal azotemia, serum BUN:creatinine < 15.

42
Q

In late cases of postrenal azotemia, tubular function is decreased;

FENa > ____% and osmolarityurine < ____ mOsm/kg.

A

In late cases of postrenal azotemia, tubular function is decreased;

FENa > 2% and osmolarityurine < 500 mOsm/kg.

(Decreased sodium reabsorbtion and decreased ability to concentrate urine)

43
Q

True/False.

Early cases of acute renal failure and postrenal azotemia are characterized by no change in tubular function (BUN:creatinine > 15; Fractional excretion of Na < 1%; osmolarityurine > 500 mOsm/kg).

A

True.

44
Q

True/False.

Late cases of acute renal failure and postrenal azotemia are characterized by no change in tubular function (BUN:creatinine > 15; Fractional excretion of Na > 1%; osmolarityurine > 500 mOsm/kg).

A

False.

Late cases of acute renal failure and postrenal azotemia are characterized by a decrease in tubular function (BUN:creatinine < 15; Fractional excretion of Na > 2%; osmolarityurine < 500 mOsm/kg).

45
Q

A patient presents with acute renal failure (azotemia, oliguria), decreased BUN reabsorption (serum BUN:creatinine < 15), fractional excretion of sodium > 2%, and urine < 500 mOsm/kg.

Identify which of the following could potentially match the diagnosis:

Prerenal azotemia

Early postrenal azotemia

Late postrenal azotemia

Acute tubular necrosis

A

​Late postrenal azotemia;

acute tubular necrosis

46
Q

Name three causes of acute renal failure due to intrarenal azotemia.

A

(1) Acute tubular necrosis
(2) Acute interstitial nephritis
(3) Renal papillary necrosis

47
Q

What etiology is the most common cause of acute renal failure?

A

Acute tubular necrosis

48
Q

In acute tubular necrosis, _________ epithelial cells obstruct the tubules and _________ GFR.

A

In acute tubular necrosis, necrotic epithelial cells obstruct the tubules and decrease GFR.

49
Q

What is seen in the urine in cases of acute tubular necrosis?

A

Brown, granular casts

50
Q

The etiology of acute tubular necrosis is typically either ______mic or ______xic.

A

The etiology of acute tubular necrosis is typically either ischemic or nephrotoxic.

51
Q

Ischemic acute tubular necrosis is often preceded by what form of acute renal failure?

A

Prerenal azotemia

52
Q

Which two portions of the nephron are most susceptible to damage in cases of ischemic acute tubular necrosis?

A

(1) Proximal tubule
(2) Medullary segment of the thick ascending limb

53
Q

If a case of acute tubular necrosis is not due to ischemic conditions, it is likely due to what?

A

Nephrotoxic agents

54
Q

Which portion of the nephron is particularly susceptible to damage in cases of nephrotoxic acute tubular necrosis?

A

The proximal tubule

55
Q

Name a few causes of nephrotoxic acute tubular necrosis.

(Most common first)

A

Aminoglycosides;

heavy metals;

myoglobinuria (e.g. from crush injuries);

ethylene glycol;

radiocontrast dye;

urate (tumor lysis syndrome)

56
Q

How can acute tubular necrosis due to tumor lysis syndrome (elevated serum urate) be prevented prior to initiating chemotherapy?

A

Hydration;

allopurinol administration

57
Q

Describe the lab results seen in acute tubular necrosis:

Casts:

Elevated serum substances:

Serum pH:

Urine frequency:

A

Describe the lab results seen in acute tubular necrosis:

Casts: Brown, granular

Elevated serum substances: BUN, creatinine, potassium

Serum pH: metabolic acidosis

Urine frequency: oliguria

58
Q

The oliguria associated with acute tubular necrosis can last 2 - 3 ________ before recovery.

A

The oliguria associated with acute tubular necrosis can last 2 - 3 weeks before recovery.

(Tubular cells are stable cells that take time to recover.)

59
Q

A patient presents with the following lab and clinical results after starting chemotherapy:

Casts: Brown, granular

Elevated serum substances: BUN, creatinine, potassium

Serum pH: metabolic acidosis

Urine frequency: oliguria

What is the diagnosis?

A

Acute tubular necrosis

(due to the elevated urate associated with tumor lysis syndrome)

60
Q

True/False.

Acute tubular necrosis is typically irreversible, although not likely to be fatal if not treated.

A

False.

Acute tubular necrosis is typically reversible, although *dialysis is often required* *to prevent fatality due to electrolyte imbalances*.

61
Q

What are the most common causes of acute interstitial nephritis?

A

Drug-induced hypersensitivity

62
Q

Acute interstitial nephritis typically affects what part(s) of the kidney?

A

The interstitium and tubules

63
Q

What medications are the most common causes of acute interstitial nephritis?

A

NSAIDs;

penicillin;

diuretics

64
Q

How is acute interstitial nephritis typically treated?

A

Typically resolves with cessation of the inducing medication

65
Q

Acute interstitial nephritis may progress to what?

A

Renal papillary necrosis

66
Q

What are the S/Sy of acute interstitial nephritis?

A

Oliguria;

fever;

rash

67
Q

What may be seen in the urine in cases of acute interstitial nephritis?

A

Eosinophils

68
Q

Acute interstitial nephritis is typically seen how long after starting a medication (e.g. NSAIDs, penicillin, or diuretics)?

A

Days to weeks

69
Q

Name a few common etiologies of renal papillary necrosis (most important will be bolded).

A

(1) Chronic analgesic use
(2) Diabetes mellitus
(3) Sickle cell (trait or disease)
(4) Severe acute pyelonephritis

70
Q

What are some of the S/Sy of renal papillary necrosis?

A

Gross hematuria;

flank pain

71
Q

Which is the most common of the three congenital cystic renal diseases?

A

ADPKD

72
Q

ARPKD is caused by a mutation in the ______ gene on chromosome 6.

A

ARPKD is caused by a mutation in the PKHD1 gene on chromosome 6.

73
Q

Which of the congenital cystic kidney diseases presents in infants as hypertension, lung hypoplasia, and dilated medullary and cortical collecting ducts?

A

ARPKD

74
Q

Which is the most common cystic renal disease in children?

A

Multicystic dysplastic kidney disease

75
Q

What is the histological hallmark of multicystic dysplastic kidney disease?

A

Ducts and tubules surrounded by cuffs of cellular mesenchyme

76
Q

True/False.

Renal cysts are very common.

A

True.

77
Q

Name the most common ureteral anomaly.

A

Double and/or bifid ureter

78
Q

Describe the histology of extrophy of the bladder.

A

Squamous or colonic glandular metaplasia

(Increased risk of adenocarcinoma)

79
Q

What is the most common cause of bladder outlet obstruction in male infants?

A

Posterior urethral valves